Cases reported "Nose Neoplasms"

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1/94. Transnasal endoscopic repair of congenital defects of the skull base in children.

    OBJECTIVE: To examine imaging findings and methods of endoscopic treatment of congenital skull base defects in children. DESIGN: Retrospective study and case series. SETTING: Academic tertiary care center. patients: Four patients (aged 12 and 14 months and 8 and 13 years) were included from 1995 to 1997. Three presented with a nasal glioma, which was recurrent in 1 case. The fourth patient presented with bacterial meningitis due to a spontaneous cerebrospinal fluid leak. Computed tomography and magnetic resonance imaging were used to locate the defect of the skull base. INTERVENTION: Transnasal endoscopic resection of the glioma or the meningocele, with immediate repair of the skull base defects using free mucosal flaps and/or pediculized mucosal flaps and/or conchal cartilage together with fibrin glue and nasal packing during a 3-week period. RESULTS: None of the 4 patients has experienced recurrent cerebrospinal fluid leaks or postoperative meningitis. CONCLUSIONS: The transnasal endoscopic repair of congenital meningoceles is a reliable technique in select pediatric patients. Computed tomography and magnetic resonance imaging provide information that can be used to help the surgical procedure.
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ranking = 1
keywords = mucosa
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2/94. Mucocutaneous angiomyolipoma. A report of 2 cases arising in the nasal cavity.

    OBJECTIVE: angiomyolipoma (AML) is a distinctive tumor that usually occurs in the kidney and rarely in the liver. angiomyolipoma of other sites is extremely rare, and, to our knowledge, only 2 cases have been described in the nasal cavity. We present 2 additional cases of AML of the nasal cavity and discuss the difference between them and renal AML. case reports AND PATHOLOGIC FINDINGS: Two tumors in a 66-year-old man and an 88-year-old woman without tuberous sclerosis are described. They showed 20-mm, well-circumscribed, polypoid shapes. Histologically, they were composed of mature smooth muscle cells, fat cells, and various-sized blood vessels. In addition, aggregated small lymphocytes were noted. Neither epithelioid smooth muscle cells nor HMB45 immunoreactivity was seen in either case. CONCLUSIONS: The clinicopathologic features of AML of the nasal cavity are distinct from renal and hepatic AML and are common to those of AML arising in the skin and oral and pharyngeal mucosa. The term mucocutaneous angiomyolipoma is thought to be appropriate to express these characteristic tumors.
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ranking = 0.5
keywords = mucosa
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3/94. MALT lymphoma of nasal mucosa treated with antibiotics.

    Mucosa associated lymphoid tissue (MALT) lymphomas occur in sites other than the gastrointestinal tract and in the early stages respond to treatment with antibiotics. We report a rare case of nasal mucosal MALT lymphoma which responded to treatment with antibiotics and has since then remained in remission.
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ranking = 2.5
keywords = mucosa
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4/94. Angiectatic nasal polyps that clinically simulate a malignant process: report of 2 cases and review of the literature.

    BACKGROUND: Approximately 5% of inflammatory or allergic sinonasal polyps develop extensive vascular proliferation and ectasia with deposition of pseudoamyloid. These so-called angiectatic nasal polyps (ANPs) can grow rapidly and exhibit an aggressive clinical behavior that could simulate malignancy preoperatively. OBJECTIVE: To systematically address the differential histologic diagnosis of ANPs. methods: We evaluated by light microscopy, immunohistochemistry, and electron microscopy biopsy and resection specimens from 2 large ANPs (8 and 10 cm in diameter) that presented in 2 adult men with life-threatening epistaxis and facial deformity, respectively. RESULTS: The tumors were firm, lobulated, and covered by smooth, partially ulcerated mucosa. Histologically, clusters of dilated, thin-walled blood vessels embedded in pools of congo red-negative eosinophilic material, associated with patchy necrosis and atypical stromal spindle cells, were seen. Electron microscopy and immunohistochemistry (CD34, factor viii) confirmed the endothelial nature of the cells lining the spaces, whereas the atypical stromal cells were classified as myofibroblasts. CONCLUSIONS: These 2 cases represent extreme examples of ANPs that clinically simulate a malignant process. awareness of the histological features of ANPs should prevent confusion of such lesions with other vascular or spindle cell lesions of the nasopharynx that would require different treatment and carry a different prognosis.
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keywords = mucosa
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5/94. Olfactory neuroepithelioma arising from the olfactory placode.

    The patient was a 54-year-old man, who had lost his sense of smell 6 years previously and had started to become forgetful about 6 months prior to presenting at hospital. MRI admission showed a large multicystic tumor with Gd-DTPA enhancement extending from the anterior cranial fossa through the sphenoid sinus and into the nasal cavity. Histopathological examination revealed extensive proliferation of small round cells that were divided by connective tissue septae. The tumor cells occasionally formed tubular structures, although no basement membranes were present. On immunostaining, round tumor cells were positive for neuron-specific enolase, synaptophysin, and chromogranin a, while cells forming tubules were positive for AE 1 and CAM 5.2. Almost all of the tumor cells were positive for Ber-EP4, and some of the epithelioid cells surrounding the tubular structures were also positive for luteinizing hormone-releasing hormone (LH-RH). Electron microscopy demonstrated sporadic intercellular junctions, many microtubules in the tumor cell processes, and clear- and dense-cored vesicles in the cytoplasm. Based on the results, this case appears to be the first documented neuroepithelioma with Ber-EP4- and LH-RH-positive cells arising from the olfactory placode.
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ranking = 0.0057639087572435
keywords = membrane
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6/94. Radiographic features of olfactory neuroblastoma.

    Olfactory neuroblastomas are malignant, slowly growing neurogenic tumors originating from the olfactory mucosa of the nasal cavity. Fourteen patients with this tumor were evaluated and showed variable extension into adjacent structures such as the ethmoid and sphenoid sinuses, orbit, and anterior cranial cavity.
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7/94. Merkel cell carcinoma of the skin and mucosa: report of 12 cutaneous cases with 2 cases arising from the nasal mucosa.

    BACKGROUND: Merkel cell carcinoma (MCC) is an uncommon skin tumor that most frequently arises on sun-exposed facial sites. It rarely occurs on mucous membranes of the head region. The primary MCC is usually treated by wide excision followed by radiation to the primary site and regional lymph nodes. Using traditional surgery the local recurrence rate ranges from 20 to 50%. In our clinic, mohs surgery is used to excise the primary MCC completely, followed by radiation. Here we present our treatment experiences and outcomes. OBJECTIVE: To document our experience of MCC treated by mohs surgery. We present our series of 12 cases of MCC, 2 cases of which arose from mucosal sites of the nasal cavity. methods: We reviewed 12 cases of MCC from the Mohs clinic database. We also reviewed the literature for cutaneous and mucosal MCC. RESULTS: There were 12 cases of MCC: 10 cutaneous and 2 mucous. The site distribution of cutaneous MCC was eight on the head, one on the neck, and one on the groin. Of these, nine were treated by Mohs excision. Two patients developed local recurrence following Mohs treatment. The local recurrence rate was 22% (2 of 9). The sites of mucosal MCC were the nasal septum and nasopharynx. One case had a history of previous radiation and developed an MCC 40 years later. This case also demonstrated epidermotropic spread of merkel cells to the overlying mucous epithelium. This patient required extensive intranasal and cranial surgery to remove the tumor. Both patients with mucosal MCCs died of their disease. The overall mucocutaneous survival of MCC at 1 year was 80% and at 2 years was 50%. CONCLUSION: In our series, local control of the primary MCC was achieved in 70% of patients (7 of 10) using combined Mohs excision and radiation. Two recurrences had primary tumors larger than 3.5 cm in diameter, while the other case was nonresectable by mohs surgery. Tumor size appeared to determine the degree of local control. When the postoperative Mohs defect was less than 3.0 cm in diameter, local and regional control appeared to be more favorable. When the primary facial MCC is relatively small, removal by mohs surgery followed by radiation was effective, therapeutic, and less disfiguring. Mucosal MCC is rare and may occur as a long-term sequelae after radiation therapy to the skin.
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ranking = 6.0057639087572
keywords = mucosa, membrane
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8/94. Nasal angiocentric lymphoma: an entity that should be remembered.

    After four nasal aesthetic functional surgeries in a period of 18 months, a 46-year-old woman was evaluated who presented with moderate functional alteration, saddle-nose deformity, and total loss of the septal cartilage. Four months before presentation the patient sustained severe nasal trauma, resulting in depression of the nasal bridge without loss of function. Her problem was diagnosed initially as a consequence of an infected septal hematoma and loss of the septal cartilage. Based on this diagnosis, the patient was subjected, in an 18-month period, to four reconstructive surgeries by different specialists, without any improvement and with worsening of clinical presentation. During the authors' physical examination of the patient, she demonstrated marked nasal cutaneous retraction, atrophic nasal conchae with total loss of the septal cartilage, and a large loss of septal bone. Three nasal mucosa biopsies were acquired and the authors proceeded to carry out complete nasal reconstruction using external cranial table and rib cartilage. Histopathologically, a lesion was noted that was compatible with angiocentric lymphoma, for which treatment was administered according to this type of illness. The authors point out the importance of establishing an adequate diagnosis in the face of an apparently obvious clinical case, present cross-disciplinary treatment, and discuss the study protocol that should be used for this type of pathology. They present their reconstructive technique of the nasal structure using a combination of bone tissue and cartilage, the results, and the current state of the patient.
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keywords = mucosa
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9/94. Extraskeletal Ewing's sarcoma of the nose.

    A 20-year-old woman presented with nasal obstruction and slight epistaxis. The obstructing lesion was excised and microscopy showed a neoplasm composed of comparatively uniform undifferentiated cells forming solid nests. The cytoplasm of the cells was clear but poorly demarcated, partly vacuolated and contained much glycogen. Although widespread in the nasal mucosa, the cells did not penetrate into the underlying bone. The cells expressed the MIC2 gene (using the CD99 marker). Electron microscopy showed simple cells with a small number of mitochondria, many glycogen particles; there were no neurosecretory granules present. Early surgical treatment followed by chemo- and radiotherapy have greatly improved the prognosis of EWS: extraskeletal Ewing's sarcoma (EWS/PNET).
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ranking = 0.5
keywords = mucosa
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10/94. True hemangiopericytoma of the nasal cavity.

    Two cases of nasal tumors with pericytic myoid differentiation are reported. The tumors occurred in a 77-year-old woman and a 60-year-old man as polypoid lesions covered by normal mucosa. Histologically, the tumors were composed of uniform short spindle or stellate cells with indistinct cell borders arranged in narrow and short fascicles. Numerous blood vessels of various sizes were common in both cases. The tumor cells of both cases stained intensely with anti-vimentin and anti-actin antibodies, but not with anti-desmin, CD34, or anti-high-molecular-weight caldesmon antibodies. Ultrastructural examination revealed well-developed actin thin filaments with dense bodies, subplasmalemmal plaques, intercellular junctions, and irregular discontinuous basement membranes. These histopathologic features suggest true pericytic differentiation of the tumors (true hemangiopericytoma), unlike soft tissue-type hemangiopericytoma. Generally, sinonasal hemangiopericytomas are subdivided into soft tissue-type hemangiopericytomas and true hemangiopericytomas identical to the cases presented here. Soft tissue-type hemangiopericytomas are frequently highly aggressive, whereas true hemangiopericytomas show localized benign behavior. Sinonasal true hemangiopericytomas should be strictly differentiated from soft tissue-type hemangiopericytomas.
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ranking = 0.50576390875724
keywords = mucosa, membrane
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